What Is von Willebrand Disease?
Von Willebrand disease (VWD) is a bleeding disorder.
It affects your blood's ability to clot. If your blood doesn't clot, you can
have heavy, hard-to-stop bleeding after an injury. The bleeding can damage your
internal organs or even cause death, although this is rare.
In VWD, you either have low levels of a certain
protein in your blood, or the protein doesn't work the way it should. The
protein is called von Willebrand factor, and it helps the blood clot.
Normally, when one of your blood vessels is injured,
you start to bleed. Small blood cell fragments called platelets (PLATE-lets)
clump together to plug the hole in the blood vessel and stop the bleeding. Von
Willebrand factor acts like glue to help the platelets stick together and form
a blood clot.
Von Willebrand factor also carries clotting factor
VIII (8), another important protein that helps your blood clot. Factor VIII is
the protein that's inactive or missing in people who have
hemophilia,
another clotting disorder.
VWD is more common and usually milder than
hemophilia. In fact, VWD is the most common of all the inherited bleeding
disorders. It occurs in about 1 out of every 100 to 1,000 people. VWD affects
both males and females, while hemophilia mainly affects males.
Types of von Willebrand Disease
There are three major types of VWD.
Type 1
In type 1 VWD, you have a low level of von
Willebrand factor, and you may have lower than normal levels of factor VIII.
This is the mildest and most common form of VWD. About 3 out of 4 people who
have VWD have type 1.
Type 2
In type 2 VWD, the von Willebrand factor doesn't
work the way it should. Type 2 is divided into subtypes: 2A, 2B, 2M, and 2N.
Different gene mutations (changes) cause each type, and each is treated
differently. So it's important to know the exact type of VWD that you have.
Type 3
In type 3 VWD, you usually have no von Willebrand
factor and low levels of factor VIII. Type 3 is the most serious form of VWD,
but it's very rare.
Overview
Most people who have VWD have type 1, a mild form.
This type usually doesn't cause life-threatening bleeding. You may need
treatment only if you have surgery, tooth extraction, or trauma. If you need
treatment, medicines and medical therapies are used.
Some people who have severe forms of VWD need
emergency treatment to stop bleeding before it becomes life threatening.
Early diagnosis is important. With the right
treatment plan, even people who have type 3 VWD can live normal, active
lives.
What Causes von Willebrand Disease?
Von Willebrand disease (VWD) is almost always
inherited. "Inherited" means that parents pass the gene for the disorder on to
their children.
You can inherit type 1 or type 2 VWD if only one of
your parents passes the gene on to you. You usually inherit type 3 VWD only if
both of your parents pass the gene on to you. Your symptoms may be different
from your parents' symptoms.
Some people have the genes for the disorder but
don't have symptoms. However, they still can pass the genes on to their
children.
Some people develop a form of VWD later in life as a
result of other medical conditions. This form of VWD is called acquired von
Willebrand syndrome.
What Are the Signs and Symptoms of von Willebrand
Disease?
The signs and symptoms of von Willebrand disease
(VWD) depend on the type and severity of the disorder. Many people have such
mild symptoms that they don't know they have the disorder.
If you have type 1 or type 2 VWD, you may have the
following mild-to-moderate bleeding symptoms:
- Frequent, large bruises from minor bumps or
injuries
- Frequent or hard-to-stop nosebleeds
- Extended bleeding from the gums after a dental
procedure
- Heavy or extended menstrual bleeding in women
- Blood in your stools from bleeding in your
intestines or stomach
- Blood in your urine from bleeding in your
kidneys or bladder
- Heavy bleeding after a cut or other accident
- Heavy bleeding after surgery
People who have type 3 VWD may have all of the
symptoms listed above, as well as severe bleeding episodes for no reason. These
bleeding episodes can be fatal if not treated right away. People who have type
3 VWD also may have bleeding into soft tissues or joints, causing severe pain
and swelling.
Heavy menstrual bleeding is often the main symptom
of VWD in women. Doctors call this menorrhagia (men-o-RA-je-a). They define it
as:
- Bleeding with clots larger than about 1-inch in
diameter
-
Anemia
or low blood iron
- The need to change pads or tampons more than
every hour
However, just because a woman has heavy menstrual
bleeding doesn't mean she has VWD.
How Is von Willebrand Disease Diagnosed?
Early diagnosis of von Willebrand disease (VWD) is
important to make sure that you're treated and can live a normal, active
life.
Sometimes VWD is hard to diagnose. People who have
type 1 or type 2 VWD may not have major bleeding problems. As a result, they
may not be diagnosed until they have heavy bleeding after surgery or some other
trauma.
On the other hand, type 3 VWD can cause major
bleeding problems during infancy and childhood. As a result, children who have
type 3 VWD usually are diagnosed during their first year of life.
To find out whether you have VWD, your doctor will
review your medical history and the results from a physical exam and tests.
Medical History
Your doctor will likely ask questions about your
medical history and your family's medical history. He or she may ask about:
- Any bleeding from a small wound that lasted more
than 15 minutes or started up again within the first 7 days following the
injury.
- Any extended, heavy, or repeated bleeding that
required medical attention after surgery or dental extractions.
- Any bruising with little or no apparent trauma,
especially if you could feel a lump under the bruise.
- Any nosebleeds that occurred for no known reason
and lasted more than 10 minutes despite pressure on the nose, or any nosebleeds
that needed medical attention.
- Any blood in your stools for no known reason.
- Any heavy menstrual bleeding (for women). This
bleeding usually involves clots or lasts longer than 7 to 10 days.
- Any history of muscle or joint bleeding.
- Any medicines you've taken that might cause
bleeding or increase the risk of bleeding. Examples include aspirin and other
nonsteroidal anti-inflammatory drugs (NSAIDs), clopidogrel, warfarin, or
heparin.
- Any history of liver or kidney disease, blood or
bone marrow disease, or high or low blood platelet counts.
Physical Exam
Your doctor will do a physical exam to look for
unusual bruising or other signs of recent bleeding. He or she also will look
for evidence of liver disease or
anemia
(a low red blood cell count).
Diagnostic Tests
No single test can diagnose VWD. Your doctor may
recommend a combination of
blood
tests to diagnose the disorder. These tests may include:
- Von Willebrand factor antigen. This test
measures the amount of von Willebrand factor in your blood.
- Von Willebrand factor ristocetin
(ris-to-SEE-tin) cofactor activity. This test shows how well your von
Willebrand factor works.
- Factor VIII clotting activity. Some people who
have VWD have low levels of factor VIII activity, while others have normal
levels.
- Von Willebrand factor multimers. This test is
done if one or more of the first three tests are abnormal. It shows the
structure of your von Willebrand factor. The test helps your doctor diagnose
what type of VWD you have.
- Platelet function test. This test measures how
well your platelets are working.
You may have these tests more than once to confirm a
diagnosis. Your doctor also may refer you to a hematologist (a blood disease
specialist) to confirm the diagnosis and for followup care.
How Is von Willebrand Disease Treated?
Treatment for von Willebrand disease (VWD) is based
on the type of VWD you have and how severe it is. Most cases of VWD are mild,
and you may need treatment only if you have surgery, tooth extraction, or an
accident.
Medicines are used to:
- Increase the release of von Willebrand factor
and factor VIII into the bloodstream
- Replace von Willebrand factor
- Prevent the breakdown of blood clots
- Control heavy menstrual bleeding in women
Specific Treatments
Desmopressin (DDAVP) is a synthetic hormone that you
usually take by injection or nasal spray. It makes your body release more von
Willebrand factor and factor VIII into your bloodstream. DDAVP works for most
people who have type 1 VWD and for some who have type 2 VWD.
Von Willebrand factor replacement therapy is an
infusion of a concentrate of von Willebrand factor and factor VIII into a vein
in your arm. This treatment may be used if you:
- Can't take DDAVP or need extended treatment
- Have type 1 VWD that doesn't respond to DDAVP
- Have type 2 or type 3 VWD
Antifibrinolytic medicines help prevent the
breakdown of blood clots. They're mostly used to stop bleeding after minor
surgery, tooth extraction, or an injury. These medicines may be used alone or
together with DDAVP and replacement therapy.
Fibrin glue is medicine that's placed directly on a
wound to stop bleeding.
Treatments for Women
Treatments for women who have VWD with heavy
menstrual bleeding include:
- Combined oral contraceptives (birth control
pills). The hormones in these pills can increase the amount of von Willebrand
factor and factor VIII in your bloodstream and reduce menstrual blood loss.
Birth control pills are the most recommended birth control method for women who
have VWD.
- A levonorgestrel intrauterine device. This is a
contraceptive device that contains the hormone progestin. The device is placed
in the uterus (womb).
- Aminocaproic acid or tranexamic acid. These
antifibrinolytic medicines can reduce bleeding by slowing the breakdown of
blood clots.
- DDAVP.
For some women who are done having children or don't
want children, endometrial ablation is done. This procedure destroys the lining
of the uterus. It has been shown to reduce menstrual blood loss in women who
have VWD.
If you need a hysterectomy (surgical removal of the
uterus) for another reason, this procedure will stop menstrual bleeding and
possibly improve your quality of life. However, hysterectomy carries its own
risk of bleeding complications.
Living With von Willebrand Disease
If you have von Willebrand disease (VWD), you can
take steps to prevent bleeding and stay healthy. You should:
- Avoid over-the-counter medicines that can affect
blood clotting, including aspirin, ibuprofen, and other nonsteroidal
anti-inflammatory drugs (NSAIDs).
- Always check with your doctor before taking any
medicines.
- Tell your doctor, dentist, and pharmacist that
you have VWD. Your dentist can talk to your doctor about whether you need
medicine before dental work to reduce bleeding. You also may want to tell
people like your employee health nurse, gym trainer, and sports coach about
your condition.
- Consider wearing a medical ID bracelet or
necklace if you have a serious form of VWD (for example, type 3). In case of a
serious accident or injury, the health care team treating you will know that
you have VWD.
- Exercise regularly and maintain a healthy
weight. Exercise helps keep muscles flexible. It also helps prevent damage to
muscles and joints. Always stretch before exercising.
Some safe exercises and activities are swimming,
biking, and walking. Football, hockey, wrestling, and lifting heavy weights are
not safe activities if you have bleeding problems. Always check with your
doctor before starting any exercise program.
Your parents, brothers and sisters, and children
also may have VWD. Consider telling them about your diagnosis and suggesting
that they get tested too.
Pregnancy and von Willebrand Disease
Pregnancy can be a challenge for women who have VWD.
Although blood levels of von Willebrand factor and factor VIII tend to increase
during pregnancy, women who have VWD can have bleeding complications during
delivery. They also are likely to have heavy bleeding for an extended time
after delivery.
However, you can take steps to lower the risk of
complications during pregnancy. Consult a hematologist and an obstetrician who
specialize in high-risk pregnancies before you become pregnant.
A hematologist is a doctor who specializes in
treating blood diseases and disorders. An obstetrician is a doctor who treats
and provides care for pregnant women.
Consider using a medical center that specializes in
high-risk obstetrics and has a hematologist on staff for prenatal care and
delivery.
Before you have any invasive procedure, such as
amniocentesis (AM-ne-o-sen-TE-sis), discuss with your doctor whether anything
needs to be done to prevent serious blood loss.
During your third trimester, you should have blood
tests to measure von Willebrand factor and factor VIII to help plan for
delivery.
You also should meet with an anesthesiologist to
review your choices for anesthesia (AN-es-THE-ze-a) and to discuss taking
medicine to reduce your bleeding risk. The term "anesthesia" refers to a loss
of feeling and awareness. Some types of anesthesia temporarily put you to
sleep, while others only numb certain areas of your body.
With these precautions, most women who have VWD can
have successful pregnancies.
Children and von Willebrand Disease
If your child has VWD that's severe enough to pose a
major risk of bleeding, anyone who is responsible for him or her should be told
about the condition.
For example, the school nurse, teacher, daycare
provider, coach, or any leader of afterschool activities should know,
especially if your child has one of the more severe forms of VWD. This
information will help them handle the situation if your child has an injury.
Key Points
- Von Willebrand disease (VWD) is a bleeding
disorder. It affects your blood's ability to clot. This can cause heavy,
hard-to-stop bleeding after an injury.
- In VWD, you either have low levels of a certain
protein in your blood or the protein doesn't work the way it should. The
protein is called von Willebrand factor. Von Willebrand factor also carries
factor VIII.
- There are three major types of VWD: type 1, type
2, and type 3.
- VWD is almost always inherited. Parents pass the
gene for the disorder on to their children. Some people develop a form of VWD
later in life as a result of other medical conditions. This form of VWD is
called acquired von Willebrand syndrome.
- The signs and symptoms of VWD depend on the type
and severity of the disorder. Many people have such mild symptoms that they
don't know they have the disorder.
- Symptoms include frequent large bruises from
minor bumps or injuries; frequent or hard-to-stop nosebleeds; heavy bleeding
after surgery, dental work, or a cut or other accident; heavy or extended
menstrual bleeding in women; blood in your stools or urine; or bleeding in your
internal organs and joints.
- VWD is sometimes hard to diagnose. Many people
don't have major bleeding and aren't diagnosed until they have heavy bleeding
after surgery or some other trauma.
- Your doctor will diagnose VWD based on your
medical history and the results from a physical exam and tests. Early diagnosis
is important to make sure you're treated and can live a normal, active life.
- Treatments for VWD include medicines and
therapies to replace or increase the amount of von Willebrand factor in your
blood, prevent the breakdown of clots, and control heavy menstrual bleeding in
women.
- Women who have VWD also may be treated with oral
contraceptives, intrauterine devices, or a procedure that destroys the lining
of the uterus, thus reducing menstrual blood loss.
- If you have VWD, you can take steps to prevent
bleeding and stay healthy. Avoid over-the-counter medicines that can affect
blood clotting. Always check with your doctor before taking any medicines.
Consider wearing a medical ID bracelet if you have a severe form of VWD. Alert
people, such as your dentist, pharmacist, employee health nurse, gym trainer,
and sports coach, of your condition.
- Pregnancy can be a challenge for women who have
VWD. Consult a hematologist and an obstetrician who specialize in high-risk
pregnancies before you become pregnant.
- If your child has VWD that's severe enough to
pose a significant risk of bleeding, anyone who is responsible for him or her
should be told about the condition. This will help them handle the situation if
your child has an injury.
- VWD can't be cured, but it can be treated. With
the right treatment, people who have VWD can lead normal, active lives.
Links to Other Information About von Willebrand
Disease
NHLBI Resources
Non-NHLBI Resources
Clinical Trials
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